Scientific DB Project

Diagnosis, management and outcomes of MEN1-related thymic NENs in Centers of Expertise - A retrospective cohort study from the ENETS MEN1 Task Force

Thymic-MEN1

Level

: Level 3

Launch date

: 1 April 2026

Task force chairs

: ENETS MEN1 Task Force

Principle investigators

: Simona Glasberg and Jerena Manoharan

Project description

:

Background

Thymic neuroendocrine neoplasms (t-NENs) are rare but highly aggressive manifestations of Multiple Endocrine Neoplasia type 1 (MEN1), occurring in approximately 2–8% of patients. Despite their low prevalence, they account for up to 20% of MEN1-related mortality. Current knowledge regarding screening strategies, surgical management (including prophylactic thymectomy), systemic treatment, and prognosis is limited to small retrospective series. Reported 10-year overall survival ranges between 25–54%. Robust international data are lacking.

Aim

Retrospective analysis of consecutive MEN1-associated thymic NENs patients to evaluate clinical presentation, screening strategies, tumour grade and stage, treatment modalities, and survival outcomes (OS, DSS, PFS), and to identify prognostic factors, including comparison of screening-detected versus non-screening-detected tumours.

Inclusion Criteria

  • Histologically confirmed thymic NEN
  • Proven MEN1 syndrome (genetic or clinical diagnosis)
  • Diagnosis from 2000 onwards
  • Documented informed consent in accordance with local regulations
  • Participation agreement of ENETS referral centers

Methods

Anonymized patients will be documented in the ENETS academic database (Level II). All standard Level 2 variables will be completed. Additional MEN1-specific data will include

  • mutation status,
  • smoking history,
  • screening details,
  • pathology,
  • treatment strategies,
  • and follow-up.

Data will be centrally extracted and statistically analysed (descriptive statistics, Kaplan–Meier survival analysis, Cox regression). A joint publication under the ENETS umbrella is planned following study completion.

Publication Rules

Each participating institution will receive one (co-)authorship. If author numbers are limited, authorship order will be determined according to recruitment numbers and scientific contribution. The first and last authorship positions will be assigned to the coordinating PIs.

Quick guide – REDCap data entry (MEN1 Thymic study)

This document provides step-by-step guidance on data entry in REDCap, including key study-specific requirements.

Download the quick guide (PDF, 839 KB)